TOF, DILV, DORV, Damus-Kaye-Stansel- Topic 14 Flashcards
(95 cards)
1
Q
What percent of all cyanotic heart defects are TOF?
A
10%
2
Q
TOF is the most common cause of _________ (2) syndrome.
A
Blue baby
3
Q
TOF has a very high association with what condition?
A
Down’s Syndrome
4
Q
TOF involves 4 heart defects. ___ are congenital; ___ are acquired.
A
3, 1
5
Q
What are the 4 TOF defects?
A
A large VSD
Pulmonary stenosis (RVOT obstruction)
An overriding aorta
RVH
6
Q
Simply put, TOF is ……
A
an anterior-lateral displacement of the infundibular septum
7
Q
Simply put, TOF is ……
A
an anterior-lateral displacement of the infundibular septum
8
Q
Aorta and PA start as a single tube called what?
A
Truncus arteriosus
9
Q
Aorta and PA start as a single tube divided by what? What direction does it grow?
A
Spiral septum; grows down and attaches to the ventricular septum which isolates the ventricles and isolates the aorta and the pulmonary artery
10
Q
Aorta and PA start as a single tube divided by what? What direction does it grow?
A
Spiral septum; grows down and attaches to the ventricular septum which isolates the ventricles and isolates the aorta and the pulmonary artery
11
Q
What is outflow tract septation?
A
Partitioning of the outflow tract
12
Q
Truncus Arteriosus becomes….
A
Aorta
13
Q
Conus Cordis becomes….
A
Pulmonary Artery
14
Q
What creates the conus cordis?
A
Created by a septum that forms in the outflow tract from these swellings
15
Q
What creates the conus cordis?
A
Created by a septum that forms in the outflow tract from these swellings
16
Q
RSTS
A
right superior truncus swelling
17
Q
LITS
A
left inferior truncus swelling
18
Q
RDCS
A
right dorsal conus swelling
19
Q
LVCS
A
left ventral conus swelling
20
Q
LVCS
A
left ventral conus swelling
21
Q
If the spiral septum is not midline, but shifted toward the right side of the heart- What would this cause?
A
Aorta opening would be large
Pulmonary opening would be small
Spiral septum would miss the septum
22
Q
If the spiral septum is not midline, but shifted toward the left side of the heart- What would this cause?
A
Aorta opening would be small
Pulmonary opening would be too large
Spiral septum would miss the septum
23
Q
If the spiral septum is not midline, but shifted toward the left side of the heart- What would this cause?
A
Aorta opening would be small
Pulmonary opening would be too large
Spiral septum would miss the septum
24
Q
What are risk factors of TOF?
A
Increased risk during pregnancy:
- Alcoholium in the mother
- Diabetes
- Mother > 40 y/o
- Poor nutrition during pregnancy
- Rubella or other viral illnesses during pregnancy
25
What are risk factors of TOF?
Increased risk during pregnancy:
- Alcoholium in the mother
- Diabetes
- Mother > 40 y/o
- Poor nutrition during pregnancy
- Rubella or other viral illnesses during pregnancy
26
TOF w/ Pulmonary Atresia AKA
pseudotruncus arteriosus
27
Pseudotruncus Arterosus (TOF w/ PA)
Severe variant in which there is complete obstruction (atresia) of the right ventricular outflow tract (RVOT), causing an absence of the pulmonary trunk during embryonic development
28
How does blood shunt in TOF w/ PA?
Blood shunts completely from the right ventricle to the left where it is pumped only through the aorta. The lungs are perfused via extensive collaterals from the systemic arteries and sometimes also via the ductus arterosus
29
Pentalogy of Fallot
TOF with the addition of an ASD
30
TOF results in low oxygenation of blood due to what?
Mixing of blood in the LV via the VSD and preferential flow of the mixed blood through the aorta (because of the flow through the pulmonary valve)
R --> L shunt
31
What is worse in TOF w/ PA?
R --> L shunt is significantly worse
32
What might be the body's response to low saturations and decreased pulmonary blood flow?
Elevate the hematocrit, it is not uncommon for these children to have hematocrits > 50%
33
What are symptoms of TOF?
```
Low blood oxygen sat w/ or w/o cyanosis
"Pink tet" - no cyanosis
Periods of severe hypxic spells ("tet spells")
Clubbing of fingers (skin or bone enlargement around the fingernails)
Difficult feeling/failure to gain weight
Passing out
Poor development
Squatting during episodes of cyanosis
```
34
Tet Spells
Rapid, deep breathing
Fainting/loss of consciousness
cyanosis of the lips tongue and nailbeds
Irritability or uncontrolled crying
35
Tet Spells
Rapid, deep breathing
Fainting/loss of consciousness
cyanosis of the lips tongue and nailbeds
Irritability or uncontrolled crying
36
Tet Spells: What a Child Will Do
Squatting and the knee chest position will increase aortic wave reflection, increasing pressure on the left side of the heart decreasing the right to left shunt (think clamping distal to outflow)
Thus decreasing amount of deoxygenated blood entering the systemic circulation
37
Tet Spells: What a Child Will Do
Squatting and the knee chest position will increase aortic wave reflection, increasing pressure on the left side of the heart decreasing the right to left shunt (think clamping distal to outflow)
Thus decreasing amount of deoxygenated blood entering the systemic circulation
38
Blalock Taussig Shunt
Formed anastomosis between the subclavian artery and the pulmonary artery
39
Palliation: Shunts Used
B-T Shunt
| Central Shunt
40
Currently, how often are Blalock Taussig shunts performed?
Not normally performed on infants with TOF except for severe variants such as TOF w/ pulmonary atresia (pseudotruncus arteriosus)
41
Central Shunt
Ascending aorta to the main PA
42
Advantages of Central Shunt
Applicability to small children with small peripheral vessels
Prevention of distortion of pulmonary arteries
Provision if equal pulmonary blood flow to both lungs
Lower occlusion rate (compared with the CBTS or MBTS techniques)
Avoidance of subclavian artery steal
Ease of closure during corrective repair
43
When is surgery for TOF done?
When the infant is young; when the condition warrants, palliation is done (more often on TOF w/ PA)
44
What correction is preferred over palliation?
Corrective surgery and is performed in the first few months of life (Less RV hypertrophy)
45
Total repair of TOF Risk
iniitally carried high mortality risk
The risk has gone down steadily over the years
Surgey is now often carried out in infants
46
The open-heart surgery for TOF is designed to:
1. Relieve the RVOT stenosis by careful resection of the muscule
2. Repair the VSD with a Gore-TEx patch or a homograft patch
*Additional reparative or reconstructive surgery may be done on patients as required by their particular cardiac anatomy
47
Two ways of TOF Repair
Transannular vs. Non-transannular
48
Dilated-Anuerysmal TOF
TOF w/ absent pulmonary valve
49
Hypoplasia TOF
TOF w/ pulmonary atresia
50
Rastelli Procedure
RV- PA conduit
51
Why would pacing wires be needed in surgically correcting TOF?
Due to large VSD patch
52
Intracardiac incision for TOF reapir
will be a right atrotomy or right ventriculotomy- depending on the severity RVOT obstruction; tis will disrupt the conduction system
53
CPB Considerations: incision
Median sternotomy
54
CPB Considerations: Cannulation
Arterial: Aortic
Venous: Bicaval
55
CPB Considerations: Hypothermia
Mild to moderate
56
CPB Considerations: Cardioplegia
Antegrade (multiple doses due to Ao-pulmonary collateral circulation)
57
How quickly will the heart warm during surgical repiar?
Quickly; be prepared for lots of cardioplegia!
58
What hsould you be careful with during surgical repair?
Your RV was stressed pre-op
| Be careful of vasodilators post-op since low BP can worsen RV dysfunction
59
What will you need during surgical repair to keep the blood pressure up?
Ionotropes
60
What pressures will stay high post-op for a while?
CVP and RV pressures may stay high post op for a while
61
Why is ECMO a possibility after surgical repair?
Due to RV dysfunction
62
How do you deal with high hematrocrits?
know this in advance
63
Why should you be careful with MUF?
It will be easy to get the HCt at 50% + (most likely will add volume pulled off when your warming)
64
Why should you be careful with MUF?
It will be easy to get the HCt at 50% + (most likely will add volume pulled off when your warming)
65
DORV
Double outlet right ventricle
66
What is DORV?
The Aorta and Pulmonary artery both originate from the RV and blood form the LV passes across a VSD into the RV ito reach the great arteries
Pulmonary circulation is very high pressure with increased blood flow (as with a large VSD)
67
What is DORV?
The Aorta and Pulmonary artery both originate from the RV and blood form the LV passes across a VSD into the RV ito reach the great arteries
Pulmonary circulation is very high pressure with increased blood flow (as with a large VSD)
68
CT
Conal tissue
69
What is the spectrum of variation of DORV?
TOF on one end to TGA on the other end
70
Majority of cases of DORV occur with what?
Pulmonary stenosis and VSD, behaving as a severely cyanotic TOF
Classified according to the VSD location
One of the ways that DORV is diagnosed is by the location of the VSD
71
Majority of cases of DORV occur with what?
Pulmonary stenosis and VSD, behaving as a severely cyanotic TOF
Classified according to the VSD location
One of the ways that DORV is diagnosed is by the location of the VSD
72
Symptoms of DORV
Baby tires easily, especially when feeding
Bluish skin color (lips may be blue)
Clubbing (thickening of the nail beds) on toes and fingers
Failure to gain weight and grow
peripheral edema
Dyspnea
73
Complications of DORV
CHF
Pulmonary HTN
irreversible damage to the lungs due to untreated high blood pressure in the lungs
74
Types of DORV
Sub-aortic VSD
Sub-pulmonary VSD (Tausigg- Bing)
Doubly Committed VSD
Non-commited VSD
75
What is the most common type of DORV?
Sub-aortic VSD
76
Sub-aortic VSD
pathophysiology depends on the degree of PS. With PS, the pulmonary blood flow is decreased with variable cyanosis (like TOF).
In the absence of PS, the pulmonary blood flow is increased, resulting in heart failure (like a VSD)
77
Sub-Pulmonary VSD DORV AKA
Taussig -Bing
78
Sub- Pulmonary VSD DORV
PA preferentially receives LV oxygenated blood
Desaturated blood from the RV streams to the aortta9 Like with TGA)
This Taussig-Bing anomaly is a typical example of DORV with sub-pulmonary VSD
Similar to TGA
79
Doubly Committed VSD DORV
Infundibular septum is absent leaving both aortic and pulmonary valves related to the VSD
CLinical features depend on the presence or absence of pulmonary stenosis
80
Non Committed VSD DORV
The non-committed VSD is remote from the aortic and pulmonary valve
Most patients with non-committed VSD undergo single ventricular w/ palliative strategies
Univentricular appearance
Heart appearance
81
Two basic types of repair for DORV
Anatomic- restores circulation w/ two ventricles
| Univentricular- only one ventricle is functional
82
Two basic types of repair for DORV
Anatomic- restores circulation w/ two ventricles
| Univentricular- only one ventricle is functional
83
Surgery: Subaortic VSD DORV
Intra-ventricular tunnel (LV--> VSD--> Ao)
Low risk
Age 6 months
Rastelli procedure for PS
84
Intra-ventricular Tunnel: Subaortic VSD DORV
Channels (tunnel/patch) LV blood through the VSD to the oarta (LV--> VSD--> Aorta)
Uses of a patch (polytetrafluoroetylene [PTFE] that corresponds to the circumference of the aorta)
85
Intra-ventricular Tunnel: Subaortic VSD DORV
Channels (tunnel/patch) LV blood through the VSD to the oarta (LV--> VSD--> Aorta)
Uses of a patch (polytetrafluoroetylene [PTFE] that corresponds to the circumference of the aorta)
86
Surgery: Sub-pulmonary VSD (Taussig-Bing Heart) DORV
Complex intra-ventricular tunnel to Ao or PA
With infundibular resection
Close VSD to PA plus
Arterial switch procedure
87
Surgery: Doubly Committed VSD DORV
Intra-ventricular tunnel (LV--> VSD--> Aorta)
PS or obstruction of the RVOT due to the tunnel may necessitate the creation of a right ventricle outflow patch or even a Rastelli)
The VSD, which is typically large, usually does not create difficulty in channeling left ventricular blood to the oarta with an intra-ventricular tunnel
88
Surgery: Non-Committed VSD DORV
Most difficult to correct
Univentricular repair
Complex intra-ventricular tunnel to Ao or PA patch/baffle
May use of combined atrial and ventricular approaches
Fontan procedure ultimately
89
CPB Considerations DORV: Incision
Median Sternotomy
90
CPB Considerations DORV: Cannulation
Arterial: Aortic
Venous: Bicaval (except with univentricular repair)
91
CPB Considerations DORV: Hypothermia
Mild to Moderate
92
CPB Considerations DORV: Cardioplegia
Antegrade (multiple doses due to Ao-pulmonary collateral circulation)
93
CPB Considerations DORV: Cardioplegia
Antegrade (multiple doses due to Ao-pulmonary collateral circulation)
94
CPB Case Notes: DORV
Amazingly variable in length, severity, and can be difficult post-op in pressure regulation
Will resemble pump runs for TOF
Univentricular repairs will be of the Fontan Procedure nature
Depending on pre-op lung damage and pulmonary hypertension- ECMO again may be warranted
95
CPB Case Notes: DORV
Amazingly variable in length, severity, and can be difficult post-op in pressure regulation
Will resemble pump runs for TOF
Univentricular repairs will be of the Fontan Procedure nature
Depending on pre-op lung damage and pulmonary hypertension- ECMO again may be warranted
Make sure you know how you are going to deal with high hematocrits in severe cyanotic conditions
Be careful with MUF since it will be easy to get the HCt at 50% + (most likely will add volume pulled off when you're warming)
